Jumping Mechanography as a Complementary Testing Tool for Motor Function in Children with Hereditary Motor and Sensory Neuropathy
23 March 2017
03 May 2017
22 June 2017 (eFirst)
Objective This study aims to compare mechanography, measuring force in jumping, and rising, with the 6-minute walk test (6MWT) and time function tests in pediatric patients with hereditary motor and sensory neuropathies.
Methods A cohort of 23 patients performed the 6MWT and time function tests (time to run 10 m, to rise from a supine position, and to climb four stairs), as well as the chair rising test (CRT) and the single two-legged jump (S2LJ) on a mechanography ground reaction force platform. Results were correlated calculating linear regression.
Results Correlation revealed high or moderate correlation between mechanography and the 6MWT and the time function tests: S2LJ/6MWT = 0.64; CRT/6MWT = 0.52; S2LJ/rising from floor = 0.63; CRT/rising from floor = 0.67; S2LJ/10 m run = 0.74; CRT/10 m run = 0.66; S2LJ/climb four stairs = 0.56; CRT/climb four stairs = 0.47.
Conclusion Jumping mechanography is a good additional tool for the assessment of pediatric patients with Charcot-Marie-Tooth disease and might be used for primary outcome measures. It was not feasible in more advanced stages of the disease. In less disabled children, the S2LJ, which quantifies force generated by proximal and distal muscles, might be superior to other tests.
Keywordsjumping mechanography - hereditary motor and sensory neuropathy - outcome measures - chair rising test - single two-legged jump
* The authors Katharina Vill and Lena Ille contributed equally to this article.
- 1 Rossor AM, Polke JM, Houlden H, Reilly MM. Clinical implications of genetic advances in Charcot-Marie-Tooth disease. Nat Rev Neurol 2013; 9 (10) 562-571
- 2 Fridman V, Bundy B, Reilly MM. , et al; Inherited Neuropathies Consortium. CMT subtypes and disease burden in patients enrolled in the Inherited Neuropathies Consortium natural history study: a cross-sectional analysis. J Neurol Neurosurg Psychiatry 2015; 86 (08) 873-878
- 3 (CMT-NET) DdC-M-TNN. Biomarker und Validierung von ausgewählten Ergebnismessungen in einer klinischen Beobachtungstudie an erwachsenen und jungen CMT Patienten unter Einbeziehung der CMT Gewebesammlung. Available at: https://www.viomedo.de/klinische-studien/7234/charcot-marie-tooth-natural-history-study-biomarker-validierung-ausgewaehlten-ergebnismessungen-klinischen-beobachtungsstudie-erwachsenen-jungen-cmt-patienten-einbeziehung-cmt-gewebesammlung . Accessed June 14, 2017
- 4 Gess B, Baets J, De Jonghe P, Reilly MM, Pareyson D, Young P. Ascorbic acid for the treatment of Charcot-Marie-Tooth disease. Cochrane Database Syst Rev 2015; (12) CD011952
- 5 Pharnext announces initiation of phase 3 extension study PLEO-CMT-FU of PXT3003 for treatment of Charcot-Marie-Tooth disease type 1A [news release]. Paris, France: Pharnext; December 20, 2016. Available at: https://www.pharnext.com/en/products/pxt3003 . Accessed June 14, 2017
- 6 Shy ME, Blake J, Krajewski K. , et al. Reliability and validity of the CMT neuropathy score as a measure of disability. Neurology 2005; 64 (07) 1209-1214
- 7 McDonald CM, Henricson EK, Abresch RT. , et al; PTC124-GD-007-DMD Study Group. The 6-minute walk test and other endpoints in Duchenne muscular dystrophy: longitudinal natural history observations over 48 weeks from a multicenter study. Muscle Nerve 2013; 48 (03) 343-356
- 8 Markovic G, Dizdar D, Jukic I, Cardinale M. Reliability and factorial validity of squat and countermovement jump tests. J Strength Cond Res 2004; 18 (03) 551-555
- 9 Tsubaki A, Kubo M, Kobayashi R, Jigami H, Takahashi HE. Normative values for maximum power during motor function assessment of jumping among physically active Japanese. J Musculoskelet Neuronal Interact 2009; 9 (04) 263-267
- 10 Veilleux LN, Rauch F. Reproducibility of jumping mechanography in healthy children and adults. J Musculoskelet Neuronal Interact 2010; 10 (04) 256-266
- 11 Matheson LA, Duffy S, Maroof A, Gibbons R, Duffy C, Roth J. Intra- and inter-rater reliability of jumping mechanography muscle function assessments. J Musculoskelet Neuronal Interact 2013; 13 (04) 480-486
- 12 Rittweger J, Schiessl H, Felsenberg D, Runge M. Reproducibility of the jumping mechanography as a test of mechanical power output in physically competent adult and elderly subjects. J Am Geriatr Soc 2004; 52 (01) 128-131
- 13 Buehring B, Belavy DL, Michaelis I, Gast U, Felsenberg D, Rittweger J. Changes in lower extremity muscle function after 56 days of bed rest. J Appl Physiol (1985) 2011; 111 (01) 87-94
- 14 Busche P, Rawer R, Rakhimi N, Lang I, Martin DD. Mechanography in childhood: references for force and power in counter movement jumps and chair rising tests. J Musculoskelet Neuronal Interact 2013; 13 (02) 213-226
- 15 Lang I, Busche P, Rakhimi N, Rawer R, Martin DD. Mechanography in childhood: references for grip force, multiple one-leg hopping force and whole body stiffness. J Musculoskelet Neuronal Interact 2013; 13 (02) 227-235
- 16 Fricke O, Weidler J, Tutlewski B, Schoenau E. Mechanography--a new device for the assessment of muscle function in pediatrics. Pediatr Res 2006; 59 (01) 46-49
- 17 Rittweger J, Felsenberg D, Maganaris C, Ferretti JL. Vertical jump performance after 90 days bed rest with and without flywheel resistive exercise, including a 180 days follow-up. Eur J Appl Physiol 2007; 100 (04) 427-436
- 18 Fricke O, Witzel C, Schickendantz S, Sreeram N, Brockmeier K, Schoenau E. Mechanographic characteristics of adolescents and young adults with congenital heart disease. Eur J Pediatr 2008; 167 (03) 331-336
- 19 Ward KA, Das G, Berry JL. , et al. Vitamin D status and muscle function in post-menarchal adolescent girls. J Clin Endocrinol Metab 2009; 94 (02) 559-563
- 20 Pouliot-Laforte A, Veilleux LN, Rauch F, Lemay M. Validity of an accelerometer as a vertical ground reaction force measuring device in healthy children and adolescents and in children and adolescents with osteogenesis imperfecta type I. J Musculoskelet Neuronal Interact 2014; 14 (02) 155-161
- 21 Opper EW, Wagner M, Bös K. , et al. Motorische Leistungsfähigkeit und körperlich-sportliche Aktivität von Kindern und Jugendlichen in Deutschland. Motorik-Modul (MoMo) im Rahmen des Kinder- und Jugendgesundheitssurveys (KiGGS). Available at: http://edoc.rki.de/oa/articles/reEzagAaWOvY/PDF/29mACsWslRxbQ.pdf . Accessed June 14, 2017
- 22 Runge M, Rittweger J, Russo CR, Schiessl H, Felsenberg D. Is muscle power output a key factor in the age-related decline in physical performance? A comparison of muscle cross section, chair-rising test and jumping power. Clin Physiol Funct Imaging 2004; 24 (06) 335-340
- 23 ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 2002; 166 (01) 111-117
- 24 Bartels B, de Groot JF, Terwee CB. The six-minute walk test in chronic pediatric conditions: a systematic review of measurement properties. Phys Ther 2013; 93 (04) 529-541
- 25 Gilchrist LS, Tanner L. The pediatric-modified total neuropathy score: a reliable and valid measure of chemotherapy-induced peripheral neuropathy in children with non-CNS cancers. Support Care Cancer 2013; 21 (03) 847-856
- 26 Burns J, Ouvrier R, Estilow T. , et al. Validation of the Charcot-Marie-Tooth disease pediatric scale as an outcome measure of disability. Ann Neurol 2012; 71 (05) 642-652